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Surgery for congenital heart disease has advanced significantly in the past 50 years, such that repair of "simple" lesions, such as atrial septal defect, ventricular septal defect, and coarctation of the aorta carries minimal risk, with mortality risk much less than 1%. It was once thought successful repair of these lesions was definitively corrective. There is mounting evidence, however, that there are long-term complications after these repairs, prompting the need for continued follow-up. This review describes the current understanding of diagnosis, treatment, and long-term outcomes for these patients, with the goal of advocating for lifelong surveillance. As the perioperative care of these repairs has evolved significantly over time, so must the way in which we study these patients in the long-term.
Obesity affects adults with congenital heart disease (CHD). The effect of an increased body mass index (BMI) with respect to morbidity and mortality has not been evaluated in adults with complex CHD. Our objective was to evaluate the effects of increased BMI on heart failure and mortality in univentricular patients who had undergone Fontan palliation.
A query of Fontan patients' first appointments at the Washington University Center for Adults with CHD between 2007 and 2014 yielded 79 patients. BMI status as normal (<25 kg/m2), overweight (≥25, <30 kg/m2), and obese (≥30 kg/m2) was established at the patient's first appointment. We analyzed demographics, diuretic requirements, New York Heart Association (NYHA) class, and laboratory values using Student's two-sample t-test and Fisher's exact test. Mortality was assessed via survival curves, and hazard ratios were compiled with proportional hazard modeling.
The recent average BMI was significantly greater in patients with NYHA classes II–IV (29.3 ± 9 kg/m2) compared with asymptomatic patients (24.8 ± 5.1 kg/m2, P = .006). Additionally, the average BMI of patients with a high diuretic requirement (≥40 mg/day IV furosemide equivalent) was obese, at 32.15 ± 9.1 kg/m2, compared with 25.91 ± 7.3 kg/m2 for those on no or lower doses of diuretics (P = .009). Eighteen of the 79 patients met an endpoint of death, hospice placement, or cardiac transplant by the study conclusion. Kaplan–Meier analysis from time of first appointment until recent follow-up revealed a significant association between time to combined endpoint and BMI class. Cox proportional hazard modeling with age adjustment yielded a hazard ratio of 3.2 (95% CI 1.096–9.379) for obesity upon first presentation to an adult CHD clinic.
In patients with univentricular hearts and Fontan palliation, obesity is associated with symptomatic heart failure and mortality.
The objective of this study was to investigate variation in practice surrounding elective cardiac catheterization in patients with single-ventricle (SV) congenital heart disease.
Patient and procedural characteristics and outcomes during SV catheterization were collected prospectively from eight centers using a web-based registry (Congenital Cardiac Catheterization Project on Outcomes). We attempted to identify a population of elective procedures by limiting the cohort in case type and timing. Cases were then stratified by stage of SV palliation (pre-bidirectional cavopulmonary anastomosis [pre-BCPA], pre-Fontan and post-Fontan) and limited by age. Subcohort analysis was performed by mode of airway management (assisted vs. spontaneous ventilation). Institutional variation was assessed.
Between 2/2007 and 6/2010, 1459 (10.1%) of 14 467 cases in the registry met the inclusion and exclusion criteria, including 326 pre-BCPA, 571 pre-Fontan and 562 post-Fontan procedures. Median patient age was 0.4 (interquartile range 0.3, 0.5), 2.6 (1.0, 3.4) and 9.6 (5.2, 15.4) years and weight was 5.6 (4.8, 6.4), 12.2 (10.5, 14), and 26.3 (16.6, 51.8) kg in the pre-BCPA, pre-Fontan and post-Fontan cohorts, respectively. Cases were more commonly diagnostic in the pre-BCPA cohort (57%) whereas they were more commonly interventional in the pre-Fontan (69%) and post-Fontan (77%) cohorts. At least one adverse event (AE) occurred in 210 cases (14.4%) overall, including 20% of pre-BCPA, 11% of pre-Fontan and 14% of post-Fontan catheterizations. Mode of airway management was associated with statistically significant, but clinically small differences in hemodynamic measures in the pre- and post-Fontan cohorts, but not in the pre-BCPA group. Considerable practice variation exists across centers with variability in airway management, AE rate, case type, interventions performed and fluoroscopy time, in all SV cohorts.
Elective catheterization in SV patients, frequently performed with intervention, is common among US congenital cardiac centers. However, important variation in practice exists during these procedures in SV patients, at all stages of palliation.
Arterial switch operation has become the treatment of choice for neonates with transposition of the great arteries. The most important step of the procedure is transferring the coronary arteries to the neoaorta successfully. This study shows the impact of coronary anatomy on early mortality and morbidity after arterial switch operation.
Ninety-two patients with transposition of the great arteries who underwent arterial switch operation between October 2010 and September 2014 were included in this retrospective study. The patients were classified into two groups: group I (n = 68, patients with usual coronary artery anatomy) and group II (n = 24, patients with unusual coronary artery anatomy). Median age was 10 days (6–25 days) in group I and 14 days (7–29 days) in group II. In group I, 25 patients had ventricular septal defect, nine patients had coarctation of the aorta or distal aortic arch hypoplasia, seven patients had Taussig Bing anomaly. In group II, nine patients had ventricular septal defect, one patient had coarctation of aorta, and one patient had Taussig Bing anomaly.
Regarding the postoperative variables, no significant statistical difference was found between two groups. But cardiopulmonary bypass time is significantly longer in group II (P = .004). There was no difference in complications including the mortality (P = .265). It is statistically found that associated anomalies did not affect the mortality and complication rates. All mortality cases (n = 4) were related to ventricular dysfunction in group II, whereas only four of the eight patients died because of ventricular dysfunction in group I.
Coronary artery pattern was not a predictor of mortality in early postoperative period. There may be an impact of unusual coronary artery pattern on the development of ventricular dysfunction as a cause of mortality.
Left axis deviation (LAD) on the electrocardiogram (ECG) is associated with congenital heart disease (CHD), prompting the clinician to order further testing when evaluating a patient with this finding. The purpose is to (1) compare the physical examination (PE) by a pediatric cardiologist to echocardiogram (ECHO) findings in patients with LAD on resting ECG and (2) assess cost of performing ECHO on all patients with LAD on ECG.
An IRB approved, retrospective cohort study was performed on patients with LAD (QRS axis ≥0° to −90°) on ECG between 01/02 and 12/12. Inclusion criteria: age >0.25 and <18 years, non-postoperative, and PE and ECHO by pediatric cardiologist. A decision tree model analyzed cost of ECHO in patients with LAD and normal/abnormal PE. Cost of complete ECHO ($239.00) was obtained from 2014 Medicare reimbursement rates.
A total of 146 patients met inclusion criteria with 46.5% (68) having normal PE and ECHO, 1.4% (2) having normal PE and abnormal ECHO, 47.3% (69) having abnormal PE and ECHO, and 4.8% (7) having an abnormal PE and normal ECHO. Sensitivity and specificity of PE for detecting abnormalities in this population was 97% and 90%. Positive and negative predictive value of PE was 91% and 97.5%. In patients with normal PE, the cost to identify an ECHO abnormality was $8365, and $263 for those with abnormal PE.
In presence of LAD on ECG, the sensitivity, specificity, and positive and negative predictive values of PE by a pediatric cardiologist are excellent at identifying CHD. Performing an ECHO on patients with LAD on ECG is only cost effective in the presence of an abnormal PE. In the presence of normal PE, there is a possibility of missing incidental structural cardiac disease in approximately 2% if an ECHO is not performed.
Critically ill children with congenital heart disease (CHD) are at risk for metabolic bone disease (MBD) and bone fractures. Our objective was to characterize a cohort of CHD patients with fractures and describe a Fragile Bone Protocol (FBP) developed to reduce fractures.
Patients who developed fractures in the Cardiac Intensive Care Unit (CICU) of Boston Children's Hospital from 3/2008 to 6/2014 were identified via quality improvement and radiology databases. The FBP (initiated July 2011) systematically identifies patients at risk for MBD and prescribes special handling precautions.
Twenty-three fractures were identified in 15 children. Median age at fracture identification was 6.2 months, with a median duration of hospitalization before fracture diagnosis of 2.7 months. Six patients (40%) had single ventricle CHD. Hyperparathyroidism and low 25-OH vitamin D levels were present in 77% and 40% of those tested, respectively. Compared with patients not diagnosed with fractures, fracture patients had increased exposure to possible risk factors for MBD and had elevated parathyroid and decreased calcitriol levels.Six patients (40%) did not survive to hospital discharge, compared with an overall CICU mortality rate of 2.6% (P < .01). The fracture case rate before implementation of the FBP was 2.6 cases/1000 admissions and was 0.7/1000 after implementation of the FBP (P = .04).
Critically ill CHD patients are at risk for fractures. They represent a complex group who frequently has hyperparathyroidism and decreased calcitriol levels, and each may predispose to fractures. FBPs consisting of identification and careful patient handling should be considered in at-risk patients.
The feasibility and safety of pregnancy after the Fontan operation is not well understood. We sought to determine contraception practices and early and late outcomes of pregnancy after the Fontan operation.
We performed a retrospective review of medical records to identify women of childbearing age from the Mayo Clinic Fontan database. A follow-up questionnaire was mailed to all patients not known to be deceased at the time of study. Patients with available contraception and pregnancy data were included in the study.
Of the 138 women with available contraception data, 44% used no contraception, 12% each used barrier methods, combination hormone therapy or sterilization, 8% used Depo-Provera, 7% had intrauterine devices, 4% had a partner with a vasectomy and 1% used progestin pills. Six women had thrombotic complications (only one using oral contraceptives). Thirty-five women had pregnancy data available. Prior to the Fontan operation there were 10 pregnancies (8 miscarriages, 2 therapeutic abortions, and no live births). After the Fontan operation there were 70 pregnancies resulting in 35 miscarriages (50%), 29 live births (41%), and 6 therapeutic abortions (9%). There were no maternal deaths during pregnancy. During long-term follow up (26 ± 6 years since the Fontan), 1 death, and 1 cardiac transplant occurred. Mean gestational age of the newborns (n = 22/29) was 33.1 ± 4.0 weeks; mean birth weight (n = 20/29) was 2086 ± 770 g. There was 1 neonatal death because of prematurity and two children were born with congenital heart disease (one patent ductus arteriosus and one membranous ventricular septal defect).
Pregnancy after the Fontan operation is associated with a high rate of miscarriages, preterm delivery, and low birth weight. Further studies are needed to identify specific variables influencing risk stratification of pregnancy in this patient population.
In order to determine the feasibility of tracking quality of care in adults with congenital heart disease (ACHD), we aimed to estimate the availability of relevant data in electronic medical records (EMR) used in North American ACHD centers.
Previously proposed quality indicators (QIs) were reviewed to consider what types of data would be required for each. ACHD program directors were surveyed about the nature of electronic data in existing EMRs. From the survey, the availability of data types needed for the denominator and numerator of each QI were estimated, and an overall estimate of data availability was calculated for each QI. These estimates were adjusted by the sensitivity of identifying the patients through administrative codes. Analysis was repeated for scenarios in which various data type estimates were hypothetically dropped by half to determine the overall impact of each data type.
A total of 64 ACHD program directors responded to the survey. Of 55 QIs, average estimated data availability was 67%. QIs for tetralogy of Fallot had the highest estimated data availability (mean 88%), whereas those for atrial septal defect were lowest (mean 23%), reflecting both the need for interpretation of imaging studies and the lower reliability of billing codes for identification of ACHD patients. QIs with highest estimates were based largely on administrative data, which had the biggest impact on overall estimates. QIs needing interpretation of imaging findings had the lowest estimates, as well as certain overuse measures.
For a wide range of ACHD programs, data for proposed QIs based on administrative data are most likely to be obtainable through EMR. Data related to imaging interpretation or overuse measures are least likely. Our findings can inform future efforts to establish registry efforts or data reporting tools to track these indicators.
Understanding value provides an important context for improvement. However, most health care models fail to measure value. Our objective was to categorize inpatient encounters within an academic congenital heart program based on clinical outcome and the cost to achieve the outcome (value). We aimed to describe clinical and nonclinical features associated with value.
We defined hospital encounters based on outcome per resource utilized. We performed principal component and cluster analysis to classify encounters based on mortality, length of stay, hospital cost and revenue into six classes. We used nearest shrunken centroid to identify discriminant features associated with the cluster-derived classes. These features underwent hierarchical clustering and multivariate analysis to identify features associated with each class.
We analyzed all patients admitted to an academic congenital heart program between September 1, 2009, and December 31, 2012.
A total of 2658 encounters occurred during the study period. Six classes were categorized by value. Low-performing value classes were associated with greater institutional reward; however, encounters with higher-performing value were associated with a loss in profitability. Encounters that included insertion of a pediatric ventricular assist device (log OR 2.5 [95% CI, 1.78 to 3.43]) and acquisition of a hospital-acquired infection (log OR 1.42 [95% CI, 0.99 to 1.87]) were risk factors for inferior health care value.
Among the patients in our study, institutional reward was not associated with value. We describe a framework to target quality improvement and resource management efforts that can benefit patients, institutions, and payers alike.
So-called heterotaxy affects lateralization of the thoracic and abdominal organs. Congenital malformations may be present in one of several organ systems. Cardiac involvement includes both structural and conduction abnormalities. Data regarding arrhythmias in heterotaxy come from case reports and small case series. We pooled available data to further characterize arrhythmias in heterotaxy. A systematic review of the literature for manuscripts describing arrhythmias in heterotaxy patients was conducted. Databases including PubMed, EMBASE, and Ovid were searched. Studies describing arrhythmias in patients with heterotaxy were included if they were in English and presented characteristics of the arrhythmias. Arrhythmia characteristics were abstracted and are presented as pooled data. Freedom from arrhythmia by age was then analyzed using Kaplan–Meier analysis. A total of 19 studies with 121 patients were included in the pooled analysis. Those with right isomerism were found to be more likely to have atrial flutter, atrial tachycardia, junctional tachycardia, and ventricular tachycardia. Those with left isomerism were more likely to have atrioventricular block, intraventricular conduction delay, sick sinus syndrome, and atrioventricular nodal reentry tachycardia. Median age of onset for all arrhythmias was 4 years with no difference by specific arrhythmia or isomerism. Those with right and left isomerism are at risk for different arrhythmias but are likely to develop arrhythmias at the same age. Those with left isomerism are more likely to require pacemaker placement due to atrioventricular block. Understanding these differences allows for focused surveillance of development of these arrhythmias.
The objective of this study was to characterize the natural history of metabolic uncoupling (type B hyperlactemia and hyperglycemia) following cardiopulmonary bypass (CPB), and to determine the impact of insulin therapy on time to lactate normalization in patients without low cardiac output.
The design used was a retrospective cohort study.
The study was set in a pediatric cardiac intensive care unit in a tertiary-care urban children's hospital.
All patients were aged ≤21 years admitted between 2007 and 2013 following cardiac surgery involving CPB with empiric intraoperative corticosteroids. Eligibility criteria: simultaneous hyperlactemia (≥3.5 mEq/L) and hyperglycemia (≥200 mg/dL) within 48 hours after bypass.
Exclusion criteria were evidence of low cardiac output state, diabetes or postoperative steroid administration.
Characteristics were compared between those treated with insulin and those who were not (controls).
Outcome measures used were time from admission to onset of hyperglycemia and hyperlactemia and time to resolution. Clinical outcomes included duration of mechanical ventilation, length of stay, unplanned readmission/reoperation, hypoglycemia and death.
Of the 1345 patients receiving CPB, 132 (9.8%) met inclusion criteria. Seventy-eight (59%) were treated with insulin, leaving 54 controls. Patient characteristics, surgical complexity and time to onset of hyperglycemia and hyperlactemia were similar between groups. The insulin group had a shorter duration of hyperglycemia. There was no significant difference between groups in time to lactate normalization, ventilator days, length of stay, readmission and reoperation rates. Hypoglycemia (<60 mg/dL) occurred in three patients.
In children with metabolic uncoupling after CPB, insulin use did not shorten the time to lactate normalization or alter clinical outcomes. These findings suggest that type B hyperlactemia with hyperglycemia after CPB will resolve spontaneously and does not warrant specific treatment.
Initial palliative treatment of congenital aortic valve stenosis consists of either surgical commissurotomy or transcatheter balloon valvuloplasty. The basis for selection of primary approach usually is ill defined. It was hypothesized that aortic valve characteristics may be helpful in directing choice of intervention. This study assesses the impact of valve morphology on time to reintervention after catheter- or surgical-based therapy.
A retrospective review of preprocedural echocardiographic aortic outflow characteristics was compared with outcomes of primary surgical or balloon valve interventions. Characteristics studied included (1) annular dimension; (2) leaflet number; (3) leaflet excursion; and (4) degree of leaflet coaptation. Patients included those <20 years with a primary diagnosis of aortic stenosis (AS) and no other hemodynamically significant lesions at our institutions from 2000 to 2011. A total of 102 patients were included, 31 classified as having critical and 71 as having noncritical aortic stenosis. Of the patients, 79 were male, and 50 underwent primary catheter intervention. Echo parameters were compared with procedural outcome as defined by death or need for reintervention. Receiver operator curves were utilized to determine the point within each morphologic feature where the greatest difference occurred. This was utilized as the distinguishing point within each the morphologic group. Analysis was conducted separately for critical and noncritical aortic stenosis.
Kaplan–Meier analysis demonstrated no significant difference in time to reintervention or death whether initial palliation consisted of surgical commissurotomy or balloon valvuloplasty with respect to any of the morphologic characteristics studied.
Patients with AS do equally well with surgical commissurotomy or balloon valvuloplasty as initial palliation. This holds true for those with either critical or noncritical aortic stenosis. Valve morphology did not help in selection of initial palliative strategy. Current technologies should enable an improved selection of initial palliative approach through thoughtful, randomized trials.
A recent review supports a strategy of deferring treatment of patent ductus arteriosus (PDA) in the preterm neonate until at least the second week after birth. In light of previous suggestion that later initiation of treatment may be less efficacious for closing PDAs it is reasonable to question if delayed treatment may be less effective.
We conducted a single center retrospective review of a neonatal intensive care unit database of infants ≤37 weeks gestation with the diagnosis of PDA and treated with indomethacin from 1999 to 2007. We determined gestational age (GA), timing of indomethacin initiation, and status of the PDA at hospital discharge. Treatment failure was defined as neonates requiring further intervention to close their PDA or those who died without echo-proven PDA closure.
Of the 341 infants meeting the study criteria, 77 (23%) had defined treatment failure. The failure group had a younger median GA of 25 weeks (interquartile range [IQR], 24–26) vs. 28 weeks (IQR, 26–30) for the successful group (P < .0001). The failure group had a median treatment initiation on day of life (DOL) 4 (IQR, 1–8) compared with DOL 3 (IQR, 1–6) for those in the successful group (P = .15). Taken as a whole, infants treated after DOL 5 were significantly more likely to have treatment failure (30.1% vs. 19.3% for those treated DOL 1–5, P = .03).
Our study confirms that younger GA at birth is correlated with increased likelihood of failed PDA closure. We also show a trend indicating that later initiation of treatment may decrease the chances of successfully closing a PDA. Future examination of PDA management should consider the potential unintended consequences that may accompany a delayed treatment strategy.
While well characterized in adult patients, the pattern of hypertrophy and the extent of myocardial scarring in hypertrophic cardiomyopathy (HCM) are insufficiently known. The aim of this study was to assess the hypertrophy patterns and the prevalence and clinical significance of scars in the hearts of young patients with HCM.
A retrospective analysis of the imaging findings of 38 children (aged 12.83 ± 2 years, 30 males) with HCM who underwent cardiac magnetic resonance imaging (CMR) was performed. In addition to left ventricular mass and volumes, the examinations were assessed for the pattern of hypertrophy and presence of late gadolinium enhancement (LGE). A myocardial signal intensity ≥6 standard deviations above the mean of normal myocardium defined positive LGE. Left ventricular mass index averaged 110 ± 34 g/m2. Nineteen children (50%) had diffuse septal, 13 (34%) diffuse concentric and 6 (16%) isolated basal hypertrophy. Seven children (18%) had LGE. Patients with LGE had a greater left ventricular mass index than those without (136 ± 34 g/m2 vs. 104 ± 31 g/m2, P = .025). The only two patients who presented with an episode of aborted sudden cardiac death had LGE (P = .03).
The most common hypertrophy pattern in children with HCM was diffuse septal hypertrophy. The incidence of LGE observed is lower than that reported in adults. The presence of LGE appears to confer a risk for adverse events.
The purpose of this study was to explore the transition process in congenital heart disease (CHD) care through the perceived needs and concerns of adolescents (pretransition) and the experiential insight from adults (post-transition), in order to inform future transition initiatives and information delivery methods.
In this cross-sectional study, adolescents and adults with moderate or complex CHD participated in semistructured telephone interviews. Interview transcripts were coded using NVivo qualitative data software.
Single large urban tertiary care CHD center
Adolescents (between 16 and 20 years of age) and adults (21–40 years of age) with moderate or complex CHD. Patients with global developmental delay or known chromosomal abnormalities were excluded. Twenty adolescents and 20 adults participated in the study; each interview allowed for participants to discuss issues relevant to them.
Among adolescents, half wanted more CHD information and half were concerned about becoming adults with CHD. All adolescents had access to the Internet via a mobile phone, but only 55% had searched online for CHD information. Adolescents were interested in mentorship with other adolescents (90%) and adults (60%) with CHD either in person or via protected social media. Among adults, 55% were satisfied with the amount of information they had received as adolescents. Areas in which adults would have preferred additional information include longer-term implications of CHD, employment, insurance, family planning, and mental health.
There are similarities and differences in the perceived transition needs of a diverse group of adolescents and adults with CHD. Both cohorts desire additional information during the transition process, including interest in use of a transition checklist to learn about managing their CHD. Adolescents are interested in interactions with other teens and adult mentors with CHD and are open to Internet-based interventions. Web-based initiatives could deliver CHD information and provide a platform for social media mentorship.
Percutaneous closure of perimembranous ventricular septal defect is one of the greatest challenges in interventional cardiology. The long-term follow-up data are still limited. This report presents our experience in percutaneous closure of perimembranous ventricular septal defect with modified double-disk occluder.
The study is a retrospective analysis of cases from the Changhai Hospital clinic database.
We reviewed the clinic database from December 2001 to December 2005.
We included 117 patients who underwent percutaneous closure of perimembranous ventricular septal defects.
Procedural data and complications were assessed by an expert panel of cardiologists.
Device implantation was successfully accomplished in 113 of a total 117 patients (96.6%). The mean age was 31.1 ± 12.5 years (range 2–56 years). The patients were followed for a period of 86–134 months. The diameter of defect was 2–15 mm (mean 4.9 ± 2.9 mm). The ventricular septal defect rim below the aortic valve was 1–5 mm. The mean diameter of device used was 6.9 ± 3.7 (range 4–24 mm). Four patients presented complete atrio-ventricular block within 1 week after the procedure and recovered within 3 weeks. No other patient encountered serious adverse events during the follow-up.
Percutaneous closure of ventricular septal defect is safe and effective in most selected patients with modified double-disk occluder. Additional studies with larger cohorts and longer follow-up are needed to evaluate its safety.
Adult congenital heart disease (ACHD) patients have high rates of untreated depression and anxiety disorders. We evaluated associations among self-reported depression symptoms and alcohol/smoking tobacco use.
From 2009 to 2013, 202 ACHD patients (45% male) completed questionnaires on depressive symptoms, anxiety symptoms, and substance use as part of routine clinical care. Data were collected by retrospective chart review.
Mean age was 31 ± 10 years, 21% reported often feeling depressed and 33% reported feeling nervous or anxious. Sixty-one percent of patients reported some alcohol intake; 25% reported current or previous smoking tobacco use. Patients with depressive symptoms were 3× as likely to report drinking alcohol (OR 2.89; 95% CI 1.29–6.5) and 5× more likely to report smoking tobacco use (OR 5.17; 95% CI 1.49–17.87). Fourteen percent of patients were prescribed antidepressant/antianxiety medications; 43% of patients on medication reported depressive symptoms. In patients reporting symptoms, those who consumed alcohol were less likely to be on antidepressant/antianxiety medications (21%) than those who did not consume alcohol (56%).
Self-reported depressive symptoms are associated with increased alcohol and smoking tobacco use by ACHD patients. Alcohol use may be a means of self-medicating for untreated depression, but further investigation is needed. Risk factors, including depressive symptoms and substance use, should be routinely assessed and addressed in ACHD patients.
Individuals with L-transposition of the great arteries and two normally sized ventricles are at risk for complete heart block. Little is known about the incidence of complete heart block in those with a single ventricle L-transposition of the great arteries. In this study, we compare the incidence of complete heart block in a modern cohort of patients with L-looped single ventricle anatomy to patients with L-transposition of the great arteries and two ventricles.
We conducted a retrospective cohort study of patients with L-transposition of the great arteries who were seen at Yale-New Haven Hospital between 2001 and 2013. Patients were classified as having isolated L-transposition of the great arteries (group I), L-transposition of the great arteries and major cardiac defects with two-ventricle anatomy (group II), or L-transposition of the great arteries and single ventricle anatomy (group III). We recorded the age of onset and the circumstances of CHB in each group. We calculated the incidence rate of complete heart block and compared this between the groups.
We identified 64 patients with L-transposition of the great arteries, median age of 21 years (range 6 months–52 years): 21 in group I, 15 in group II, and 28 in group III. In total, 15 subjects developed complete heart block, incidence of 21.9% and rate of 1.3% per person years. Although group III patients were significantly less likely to develop complete heart block than dual ventricle patients (7.1% vs. 33% and 40%, P = .01), this difference is not significant when only spontaneous complete heart block was analyzed (P = .16).
All patients with L-transposition of the great arteries have similar risk of spontaneous complete heart block and should be routinely screened for this complication.
Hypertrophic cardiomyopathy (HCM) is the most common heritable cardiovascular disease and a leading cause of identifiable sudden cardiac death (SCD) in the young. Herein, we sought to determine the genotype–phenotype correlations in a cohort of unrelated, genotyped patients diagnosed with HCM at a young age, as well as to characterize the differences between HCM diagnosed in adulthood and HCM diagnosed at a young age.
From 1999 to 2011, 1053 unrelated patients diagnosed with HCM were enrolled in research-based genetic testing. The electronic medical record was reviewed to identify those with HCM diagnosed at ≤21 years (N = 137, mean age at diagnosis 13.2 ± 6 years, 64% male). From this cohort of patients recruited from a tertiary care referral center, the genetic test was positive in 71 (52%), which was significantly higher than patients diagnosed >21 years (31%; P < .001). Genotype-positive patients had increased maximum left ventricular wall thickness (24.9 ± 8.0 vs. 21.6 ± 7.4 mm, P = .01) and higher incidence of reverse-curve ventricular septal morphology (71% vs. 40%, P < .001). Unrelated to genotype status, 26/137 patients (19%) experienced significant HCM-related morbidity/mortality including progressive heart failure symptoms in 12, transplantation in 4, and death in 10.
Among patients diagnosed with HCM during the first two decades of life, the yield of genetic testing is significantly higher than when diagnosed at later age. While the phenotype of young HCM patients is worse than patients whose HCM is diagnosed at later age, the phenotypes of genotype-positive and genotype-negative young patients were similar. Independent of genotype, nearly 30% of the patients with follow-up in this study had symptom progression, transplant, or death.
Shunt or conduit thrombosis in a single ventricle circuit is a life-threatening complication that requires prompt treatment to rapidly restore shunt/conduit patency. Transcatheter interventions represent an attractive alternative to systemic thrombolysis or open surgical procedures. We report our center's experience with catheter-based approaches in patients with palliated single ventricle who present with shunt/conduit thrombosis.
A retrospective review was performed of all patients with palliated single ventricle physiology who were diagnosed over a 5-year period with shunt/conduit thrombosis and received catheter-based interventions. Patients were followed up to hospital discharge.
Thirteen patients were identified that were diagnosed with thrombosis of a modified Blalock-Taussig shunt (five patients), bidirectional cavopulmonary shunt (one patient), and total cavopulmonary pathway (seven patients). Shunt/conduit thrombosis occurred both early and late after palliation surgery. Catheter-based interventions included balloon angioplasty (one patient), stent implantation (12 patients), and mechanical thrombectomy (one patient). Thrombophilia was identified in seven patients. Technical and clinical success with restoration of normal shunt flow and improvement in clinical status was achieved in 12 patients. Reversible procedure-related complications occurred in three patients with no significant sequelae.
Our experience suggests that percutaneous catheter-based interventions are safe and effective in managing shunt/conduit thrombosis in infants and children with palliated single ventricle circulation.
Epidemiologic studies have reported conflicting results regarding maternal alcohol consumption before and during pregnancy, and the risk of congenital heart defects (CHDs). However, a systematic review and meta-analysis of the association between maternal alcohol consumption and CHDs in offspring has not been conducted.
We searched MEDLINE and EMBASE for articles catalogued between their inception and February 16, 2015; we identified relevant published studies that assessed the association between maternal alcohol consumption and CHD risk. Two authors independently assessed the eligibility of the retrieved articles and extracted data from them. Study-specific relative risk estimates were pooled by random-effects or fixed-effects models.
From the 1527 references, a total of 19 case–control studies and four cohort studies were enrolled in this meta-analysis. The summary of 23 studies related to CHDs indicated an overall pooled relative risk of 1.13 (95% confidence interval: 0.96, 1.29) among mothers drinking before or during pregnancy. Statistically significant heterogeneity was detected (Q = 196.61, P < .001, I2 = 88.8%) with no publication bias (Egger's test: P = .157). We conducted stratified and meta-regression analyses to identify the origin of the heterogeneity among studies.
In summary, this meta-analysis provided no positive association between maternal alcohol consumption and risk of CHDs.
Gastrostomy tube (G-tube) placement during three-stage surgical palliation of single-ventricle cardiac physiology has been shown to improve weight gain in this population of infants who often suffer from inadequate feeding. The optimal timing of this intervention is unclear and requires further investigation.
A retrospective review of all patients who underwent G-tube placement at any stage of surgical palliation of single-ventricle physiology from January 2005 to December 2012 was performed at a single congenital cardiac surgery center. Analysis of weight gain and survival was undertaken by comparing patients who received the G-tube either less than or greater than 90 days after the first surgical stage.
Fifty-four patients were identified that met the criteria, 26 (48%) of which received the G-tube within 90 days of stage 1, while 28 (52%) patients received the tube at greater than 90 days. Percentage of weight gain at time of discharge from stage 1 was significantly higher for group B (A: median 9.9%, interquartile range [IQR] 4.9–29.8; B: median 29.0%, IQR 16.0–44.3; P = .05). However, total hospital length of stay was decreased for the patients who received G-tubes earlier (A: median 60 days, IQR 35–100; B: median 83, IQR 48–184) as was intensive care unit length of stay (A: median 27 days, IQR 13–69; B: median 48, IQR 16–119) by nearly half, although not statistically significant (P = .47). Survival to time of discharge from stage 1 surgery was not significantly different between earlier tube placements vs. later (92% vs. 100%, respectively; P = .14). Multivariable analysis found inclusion of fundoplication to predict weight gain (P = .006) at time of first discharge.
Earlier placement of G-tube may increase the rate of recovery from stage 1 of multistage palliative cardiac surgery for single-ventricle physiology. Fundoplication may improve perioperative weight gain when indicated.
Recent advances in various disciplines of medicine have significantly changed the courses following cardiac surgery in children. On-table extubation (OTE) after open heart surgery in children is evolving.
To assess the rate of postoperative complications in children extubated on table after open heart surgery.
This is a retrospective, descriptive study.
Operating room (OR) then admitted to the pediatric intensive care unit (PICU).
All pediatric patients (between 0 and 18 years) undergoing open heart surgery between January 2011 and June 2013.
On-table extubation.
Rates of immediate postoperative complications, i.e., re-intubation, significant bleeding, low cardiac output syndrome, and arrhythmia in PICU, were assessed. Data are presented as frequencies and mean ± standard deviation.
A total of 82 patients were included. Mean age at time of operation was 7.25 ± 6.6 years. Fifty-three percent (n = 44) were <5 years old and 64% (n = 53) were men. Ventricular septal defect (47%, n = 39) was the most common lesion, followed by atrial septal defect (36%, n = 30), and tetralogy of Fallot (15%, n = 12), which were repaired. Cardiopulmonary bypass and aortic cross clamp time were 72.3 ± 34.2 and 47.3 ± 27.8 minutes, respectively. The mean inotrope score was 2.66 ± 3.53. There was no mortality in the cohort, whereas 97.8% (n = 80) had no complications during PICU stay. One patient (1.1%) required re-intubation for respiratory failure and one patient (1.1%) had arrhythmia that was medically managed. The mean length of PICU stay was 1.77 ± 0.985 days.
On-table extubation in children after open heart surgery was feasible and safe in selected group of patients. There was no major complication observed in the PICU.
After Fontan operation, hemodynamically significant veno-venous collateral (VVC) vessels can lead to systemic arterial desaturation. Outcomes after embolization of VVCs have not been determined. We sought to determine the frequency of and outcomes for patients undergoing VVCs embolization after Fontan operation.
We retrospectively analyzed clinical and hemodynamic data of patients who underwent cardiac catheterization after the Fontan operation from 1995 to 2014 at Mayo Clinic. Clinical, imaging, and hemodynamic data from patients with VVCs were compared based on intervention (embolization) vs. nonintervention.
Four hundred ninety-six patients with prior Fontan operation had catheterization; 109 VVCs were identified in 72 patients (37 males, mean age 26 ± 12 years). Embolization was performed in 31/72 patients (43%). Following embolization, no improvement was demonstrated in oxygen saturation or hemoglobin concentration at ambulatory follow-up (2.5 ± 2 years), and cardiac index declined at follow-up catheterization (5.6 ± 3 years). Overall, 15 patients (21%) died at a mean of 2.8 ± 4 years after embolization. Fifty percent of deaths after embolization occurred in patients with Fontan pressures ≥18 mm Hg. Five-year survival of patients with VVCs undergoing embolization was 74% compared with 92% in those patients who did not undergo embolization (P < .01). In multivariate analysis, significant predictors of death on follow-up were embolization [hazard ratio (HR) = 9.3 (95% confidence interval [CI], 2.8–42), P = .0001], atrio-pulmonary Fontan (HR = 4.2 [95% CI, 1.4–15], P = .01), and heterotaxy (HR = 3.7 [95% CI, 1.0–15], P = .05).
We observed decreased 5-year survival in patients who had VVC embolization. Embolization of VVC in patients after Fontan should be evaluated very carefully in patients with atrio-pulmonary type Fontan, heterotaxy, and those with Fontan pressure above 18 mm Hg. These patients may benefit from the "natural" fenestration that VVCs provide.
We aimed to determine the correlation between noninvasive testing (exercise stress testing [EST] and adenosine responsiveness of accessory pathway [AP] ) and invasive electrophysiology study (EPS) for assessment antegrade conduction of the AP in Wolff–Parkinson–White syndrome.
This prospective, observational study enrolled 40 children (58% male children, median age of 13 years, and median weight of 47.5 kg) with Wolff–Parkinson–White syndrome. Conduction through the AP to a cycle length of ≤250 ms was considered rapid or high-risk; otherwise, patients were nonrapid or low-risk.
The sudden disappearance of the delta-wave was seen in 10 cases (25%) during EST. Accessory pathway was found to be high-risk in 13 cases (13/40, 32.5%) while the accessory path was identified as low-risk in 27 cases; however, six patients (15%) had blocked AP conduction with adenosine during EPS. Low-risk classification by EST alone to identify patients with nonrapid conduction in baseline EPS had a specificity of 93% and a positive predictive value of 90% (accuracy 54%). Blocked AP conduction with adenosine as a marker of nonrapid baseline AP conduction had a specificity of 93% and a positive predictive value of 84%. Finally, AP was adenosine nonresponsive in the majority of patients (28/30, 93%) with persistent delta-waves, 40% of those who had a sudden disappearance of delta-waves had an adenosine-responsive AP (P value: .028).
Abrupt loss of preexcitation during EST and blocked AP conduction with adenosine had high specificity and positive predictive value for nonrapid and low-risk antegrade conduction during baseline invasive EPS. Successful risk stratification of pediatric patients with Wolff–Parkinson–White is possible through the use of EST and the adenosine responsiveness of AP.
Nurse practitioners (NPs) have an established role for delivering competent care to patients in the primary care setting. The aim of this study was to compare satisfaction of patients managed by NPs vs. physicians in the outpatient adult congenital heart disease (CHD) clinic.
A prospective study conducted in two outpatient adult CHD clinics to assess patient satisfaction through standardized surveys. Demographic data included CHD diagnosis, age, and New York Heart Association functional class. All patients completed a Short-Form-12 to assess health status.
Of the 371 patients recruited (52% men; median age 29 years, severe CHD complexity 31.5%) and seen by NP (n = 187) or physician (n = 184), physician-managed practices had higher perceived: overall experience, courtesy of provider (P < 0.05) and confidence (trust) in provider (P < 0.1). Overall, patients reported satisfaction with an NP providing care (98%), the NP was able to effectively deal with illness/CHD (95%), and an increased chance (94%) or willingness to see an NP at a future visit. Only 73% reported an understanding of NP training and how an NP differed from a registered nurse. There was a stronger perception of how an NP differed from a physician (83%).
Patient satisfaction was high regardless of whether care was provided by physicians or NPs. However, patients appear to make distinctions in what they believe the type of care each practitioner is best at providing. Patient education regarding competence of the different health care providers may continue to improve patient satisfaction.
In adults with cardiomyopathy, tissue Doppler imaging (TDI) has been shown to correlate with left ventricular filling pressures (LVFPs) and has been advocated as a surrogate to catheterization. However, this has not been validated in children.
This is a retrospective review of children ≤18 years old with dilated, hypertrophic, restrictive, and left ventricular noncompaction cardiomyopathy who underwent cardiac catheterization within 3 months of an echocardiogram. Spearman's correlation coefficients were calculated to assess a correlation between LVFP and mitral inflow E/A ratio, lateral mitral E/E′, and septal E/E′.
Thirty-eight patients were included in the study; median age was 8.6 years old. The median LVFP was 19 mm Hg, median mean pulmonary artery pressure was 25 mm Hg, and median pulmonary vascular resistance index (PVRi) was 2.4 Wu. There was no significant correlation between LVFP or PVRi with lateral mitral E/E′ or septal E/E′. There was a positive correlation between LVFP and mitral inflow E/A ratio (rs = 0.59, P < .01). In a subgroup analysis of patients with hypertrophic or restrictive cardiomyopathy, there was a negative correlation (rs = 0.56, P = .02) between the mean pulmonary artery pressure and septal A′.
TDI measures of diastolic function are not reliable surrogates for LVFP, mean pulmonary artery pressures, and PVRi at catheterization in children.
The impact of obesity on surgical morbidity in adults with congenital heart disease is currently unknown. The aim of our study was to investigate the impact of obesity on postoperative outcomes in adults with congenital heart disease undergoing reoperation for pulmonary valve replacement.
A retrospective analysis was performed assessing the influence of obesity on surgical outcomes. Obesity was defined as a body mass index ≥30 kg/m2.
The mean body mass index of the cohort was 25.9 ± 6.9 kg/m2. The cohort included 71 patients with 17 patients (24%) being obese. There was no postoperative mortality. Obese patients had a longer hospital length of stay (6.6 vs. 4.7 days; P < .001) and increased incidence of postoperative arrhythmias (29% vs. 5.6%; P = .003) compared with nonobese patients. Multivariable analysis performed using logistic regression with backwards elimination demonstrated obesity was independently associated with hospital length of stay >5 days (odds ratio [OR] = 5.2; 95% confidence interval [CI]: 1.5–18.2, P = .01) and with increased postoperative arrhythmias (OR = 4.2; 95% CI: 1.7–40, P < .01).
Obesity is associated with increased morbidity in adults with congenital heart disease undergoing pulmonary valve replacement, including longer hospitalization and higher risk for postoperative arrhythmias.
The purpose of this study was to compare neurological development of children with cyanotic or hemodynamically impaired congenital heart disease (CHD) and healthy controls by using "Bayley Scales of Infant and Toddler Development Screening Test, Third Edition" (Bayley-III).
Children with CHD (n = 37) and healthy controls (n = 24) aged between 1 and 41 months who were admitted to the Department of Pediatric Cardiology at our university hospital were included. The participants were assessed using Bayley-III test. All patients had cyanotic or hemodynamically impaired CHD. Weight, height, body mass index (BMI), mid-arm circumference (MAC), triceps skinfold thickness (TSF), and head circumference (HC) were measured and standard deviation scores (SDSs) were determined.
SDS values of weight, height, BMI, MAC, and TSF of the patients as well as HC values were significantly lower than the control group (P < .001). Compared with controls, the patients had significantly lower mean scores in all Bayley-III subscales (P < .001). We observed similar results in Bayley-III scores including the mean values of cognitive, language, and global motor scores for the CHD patients with and without cardiac surgery (P > .05).
This study demonstrated that children with cyanotic or hemodynamically impaired CHD have delayed neurodevelopmental outcomes compared with healthy children as assessed using Bayley-III.
This study investigated the clinician practices on perioperative anticoagulation in children with prosthetic mechanical heart valves who undergo elective surgeries.
An online survey was administered to members of PediHeartNet. The survey consisted of multiple choice questions and clinical scenarios.
The study described clinical practice patterns and variables that influence the clinicians' bridging anticoagulation decisions.
Ninety-one respondents completed the survey; 68% were affiliated with university settings; 91% were pediatric cardiologists, and 49% had ≥10 years of experience in pediatric cardiology. Approximately one-half of the respondents (54%) independently provided perioperative anticoagulation management to their patients, while 46% utilized cardiac or hematology anticoagulation services. Resources that influenced bridging decisions included hematology experts (20%), American College of Chest Physicians guidelines (34%), and the clinicians' personal experience (56%). In planning for major surgeries, 47% of the respondents hospitalized patients for unfractionated heparin (UFH) and 46% prescribed outpatient low molecular weight heparin (LMWH). For minor surgeries, 58% hospitalized patients for UFH, 22% prescribed outpatient LMWH, and 17% opted out of bridging anticoagulation. Immediately after mitral valve replacement, 23% used bridging anticoagulation with UFH. When LMWH was used, there were no reports of thromboembolic complications. Major bleeding complications were rare and reported by 2% of the respondents.
This was the first documentation that clinical practice of bridging perioperative anticoagulation in children with mechanical heart valves varies widely among pediatric cardiac specialists. There is poor adoption of published guidelines and a tendency toward more conservative strategies. Further studies comparing the safety and efficacy of LMWH vs. UFH as perioperative anticoagulation agents in children with mechanical heart valves are needed to further clarify our findings. Quality assurance initiatives and education are also needed to improve guidelines adherence and standardize practice management.
Transthoracic echocardiography (TTE) plays a key role in adult congenital heart disease (ACHD). However, a significant number of studies are nondiagnostic due to poor image quality. Enhancement of the blood pool-tissue interface with contrast-enhanced TTE (CE-TTE) can improve image quality in suboptimal studies. The aim of this analysis was to evaluate feasibility and clinical utility of CE-TTE in the assessment of patients with ACHD.
A retrospective analysis of all CE-TTE performed in ACHD patients at our institution from August 2007 to May 2014 was performed. Endocardial definition scores (EDS) for each segment in the right and left ventricles were graded pre- and postcontrast imaging, as 1 = good, 2 = suboptimal, 3 = not seen. The endocardial border definition score index (EBDSI) was also calculated pre- and postcontrast imaging.
Twenty patients with ACHD had 24 CE. Summation data for all ventricular EDS for unenhanced TTE vs. CE-TTE imaging was: EDS 1 = 136 vs. 314, EDS 2 = 119 vs. 72, EDS 3 = 162 vs. 31, respectively. Wilcoxon matched-pairs rank-signed test showed a significant ranking difference (improvement) pre- and postcontrast for the combined ventricular data (P < .0001) and the individual left and right ventricular data (all P < .0001). The EBDSI for combined ventricular data using CE-TTE was significantly lower than for noncontrast imaging (1.23 ± 0.49 vs. 2.06 ± 0.62, P < .0001). There was one minor contrast adverse reaction.
CE-TTE resulted in significantly improved right and left ventricular endocardial definition and improved EDBSI. CE-TTE should be viewed as an additional imaging technique that is available to help assess patients with ACHD, especially those with nondiagnostic images.
This review synthesizes the empirical literature on outcomes and experiences of transfer and transition from pediatric to adult care for young people with congenital heart disease. A systematic review of papers published between January 2001 and May 2013 that examined outcomes or experiences of transfer and transition among young people with congenital heart disease was conducted. Data were extracted by two independent reviewers with the outcomes data combined using narrative synthesis and the experiences data integrated using thematic synthesis. Thirteen papers were included in the review: six reported outcomes following transfer, six reported experiences of transfer and transition, and one reported both outcomes and experiences. The review data indicate that high proportions of young people were lost to follow-up or experienced long gaps in care after leaving pediatric cardiology. Factors that protected against loss to follow-up or lapse in care included: beliefs that specialized adult care was necessary; poorer health status; attendance at pediatric appointments without parents; and pediatric referral to an adult congenital heart disease center. Data on experiences highlighted that many young people were unconcerned about transition, but lacked knowledge about their condition and were insufficiently prepared for transfer. In terms of adult services, many young people desired continuity in the quality of care, youth-oriented facilities, a personalized approach, and for their parents to remain involved in their care, but in a secondary, supportive capacity.
In conclusion, the high proportions of young people lost to follow-up highlight the need for formal transition programs, which ensure a planned and coordinated transfer. Patients with congenital heart disease need education throughout adolescence about the implications of their condition, the differences between pediatric and adult services, and self-care management.
In patients with systemic right ventricles (RVs) in a biventricular circulation, exercise capacity and RV function often deteriorate over time and echocardiographic assessment of systemic RV function is difficult. The purpose of this study was to examine the relationship between exercise capacity and RV function and to determine which noninvasive imaging parameters correlate most closely with exercise capacity.
Patients with a systemic RV (D-loop transposition of the great arteries [TGA] after atrial switch procedure or physiologically "corrected" TGA) who underwent cardiopulmonary exercise testing (CPX) and noninvasive imaging (cardiac magnetic resonance [CMR] and echocardiography [echo]) within 1 year of CPX were identified. Regression analysis was used to evaluate the relationship between exercise variables and noninvasive indices of ventricular function.
We identified 92 patients with 149 encounters (mean age 31.0 years, 61% men, 70% D-loop TGA) meeting inclusion criteria. Statistically significant correlations between % predicted peak oxygen uptake (%pVO2) and RV ejection fraction (EF) (r = 0.29, P = .0007), indexed RV end-systolic volume (r = −0.25, P = .002), and Tei index (r = −0.22, P = .03) were found. In patients without additional hemodynamically significant lesions, the correlations between %pVO2 and RV EF (r = 0.37, P = .0007) and the Tei index (r = −0.28, P = .03) strengthened and a correlation emerged between %pVO2 and dP/dtic (r = 0.31, P = .007). On multivariable analysis, Tei index was the only statistically significant correlate of %pVO2 (P = .04).
In patients with systemic RVs in a biventricular circulation, CMR-derived RVEF and echo-derived Tei index correlate with %pVO2. On multivariable analysis, the Tei index was the strongest predictor of peak %pVO2 response.
Single ventricle defects are among the most complex congenital heart defects and the development of advanced surgical procedures in recent decades has created the first generation of adolescents and young adults living with this condition. Yet little is known about how these individuals experience life and what impact the heart defect has on their life in general.
The aim was to illuminate and gain a deeper understanding of adolescents' and young adults' experiences of living with a surgically palliated univentricular heart.
Seven open-ended in-depth interviews were conducted, transcribed, and analyzed according to the henomenological hermeneutical method. All adolescents and young adults operated before 1995 according to the Fontan procedure or the total cavo-pulmonary connection procedure at one pediatric cardiology unit were included in the study. They were 17–32 years of age (median age 22 years).
The interpretation of the interview transcripts showed that the participants experienced living with a surgically palliated univentricular heart in terms of feeling exceptional, strong, and healthy. This was supported by two structural analyses, where three themes emerged: happiness over being me, focusing on possibilities, and being committed to life.
Living with a Fontan circulation included negative experiences but the analyses clearly demonstrated a feeling of being strong and healthy. An appreciation of having survived and being committed to life was found to be an integral part of the development of the interviewees' existential growth. This probably strengthens them further in their ability to balance expectations and hurdles in life. This study provides valuable insights into the experience of patients after the Fontan procedure and the importance of a positive health care environment throughout their lives.
We investigated the type and extent of calcification in a series of heart specimens from adult congenital heart disease patients because recent autopsy observations suggested a high prevalence of calcification.
We used computed tomography to examine seven heart specimens from adults (>18 years old) with a congenital heart defect collected with permission from the family during a recent 3-year period. Clinical data regarding diagnosis, history, and imaging studies were recorded. The 3D data sets were reviewed after reformatting as maximum intensity projection and volumetric renderings to determine the pattern and extent of calcium deposition.
Five of the seven hearts had extensive calcifications in one or more of three patterns: atherosclerosis associated in the three oldest cases; surgery associated in four of five hearts that had undergone heart surgery; and myocardial calcification remote from surgical sites in two cases. Myocardial calcification was associated with regional dysfunction and was present in the three patients that died suddenly and unexpectedly.
Cardiac calcification was frequent in our series of heart specimens from adults with congenital heart defects, was often but not uniformly associated with prior surgery, and, in our small series, was associated with regional dysfunction and sudden death.
The purpose of this study was to determine the prevalence of growth restriction in infants and young children with congenital heart disease (CHD) and investigate the relationship between poor growth, feeding difficulties, cardiac classification, and nutrition intervention on outcomes.
This is a prospective observational cohort study of infants and young children with CHD aged 0–3 years admitted to hospital for cardiac surgery. Anthropometry, growth history, cardiac classification, cardiac diagnosis, feeding difficulty, and nutrition intervention data were collected for 78 participants.
Many participants demonstrated growth restriction as evidenced by a z-score ≤−2 for population growth parameters including weight/age z-score (n = 18, 23%), height/age z-score (n = 16, 21%), and weight/height z-score (n = 12, 18%). Increased hospital length of stay was associated with factors including faltering growth preadmission (P = .009), tube feeding required preadmission (P = .002), diagnosis of cyanotic CHD (P = .015), and presence of a feeding difficulty (P = .015).
Growth restriction remains an ongoing problem in children with CHD. Faltering growth preadmission and lower growth parameters were associated with an increased hospital length of stay. Nutritional screening from diagnosis may detect growth faltering, improve access to early nutrition intervention, and improve patient outcomes.
Adults with congenital heart disease (CHD) face increased risk for morbidity and mortality with age, but few prognostic models exist.
This study aims to assess whether the Heart Failure Survival Score (HFSS), which risk stratifies patients for heart transplantation, predicts outcomes in adults with moderate or complex CHD.
This was a multicenter, retrospective study which identified 441 patients with moderate or complex CHD between 2005 and 2013, of whom 169 had all the HFSS parameters required to calculate the risk score. Because all study patients were deemed low risk by the HFSS, the score was dichotomized at the median (10.4). Outcomes included death, transplant or ventricular assist device (VAD), arrhythmia requiring treatment, nonelective cardiovascular (CV) hospitalizations, and the composite. Associations of mean HFSS and HFSS <10.4 with each outcome were assessed.
The cohort had mean ± standard deviation age of 33.6 ± 12.6 years, peak VO2 21.8 ± 7.5 mL/kg/min, HFSS of 10.45 ± 0.88, and median years follow-up of 2.7 (1.1, 5.2). There were five deaths (2.8%), no transplants or VADs, 25 arrhythmias (14.8%), 22 CV hospitalizations (13%), and 39 composites (23.1%). Lower mean HFSS was observed for patients who died (9.6 ± 0.83 vs. 10.5 ± 0.87, P = .02), arrhythmia requiring treatment (10.0 ± 0.70 vs. 10.5 ± 0.89, P = .005), CV hospitalizations (9.9 ± 0.73 vs. 10.5 ± 0.88, P = .002), and the composite (10.0 ± 0.70 vs. 10.6 ± 0.89, P < .001). The positive and negative predictive values of HFSS <10.4 for the composite were 34% and 88% respectively, with sensitivity and specificity 74% and 56%.
Although a low HFSS was significantly associated with outcomes, it did not adequately risk stratify adults with CHD, whose heterogeneous pathophysiology differs from that of the acquired heart failure population. Further studies are warranted to provide a more accurate prognosis.
Congenital heart defects (CHDs) affect a large number of newborns and account for a high proportion of infant mortality worldwide. There are regional differences in the prevalence and distribution pattern of CHDs. The aim of this study is to estimate the distribution pattern and prevalence of CHDs among the population of north-central India and to compare the results with studies in other regions of the country to get an overview of prevalence of CHDs in India.
We carried out a prospective study in the outpatient department of a tertiary care referral center in north-central India. This study was carried out from January 2011 to April 2014, with 34 517 individuals being recruited for the study. All patients were examined by chest x-ray, electrocardiogram, and 2D echocardiography. Prevalence rate per 1000 individuals examined was calculated. Relative frequencies of individual CHD types as a proportion of total CHDs were also calculated.
Out of 34 517 individuals examined, 661 were diagnosed with CHDs, giving a prevalence of 19.14 per 1000 individuals. The most common defect was ventricular septal defect (33%), followed by atrial septal defect (19%) and tetralogy of Fallot (16%). The majority of CHD cases (58%) diagnosed were between 0 and 5 years of age. The prevalence of CHDs in adults was 2.4 per 1000 individuals in this cohort, with atrial septal defect (44.5%) being the most frequent defect.
The prevalence of CHDs in our cohort was high, possibly because of the power of the diagnostic methods we used and the inclusion of all age groups. Adults with CHDs may significantly contribute to the prevalence of CHDs in the next generation, and this needs to be considered when estimating prevalence rates. Although several small regional studies have been carried out in India, there is an urgent need to establish a nationwide registry/database for congenital heart defects.
This study was designed to compare outcomes of the most common pediatric cardiac interventions from the time of implementation with the current era.
Since the introduction of semilunar valve balloon dilation and device closure of the arterial duct and septal defects, development of interventional techniques and devices has been rapid. However, few studies have compared outcomes between those initial interventions and those in the current era.
Five validated common catheter-based therapies were chosen for analysis, including atrial and duct device closure, balloon dilation of the aortic and pulmonary valves, and native coarctation of the aorta. A retrospective review of the first and most recent 10 consecutive patients in each group was performed.
There was a high mortality (30%) among neonates who underwent aortic valve (AV) dilation in the early era, but no mortality noted in other groups. In the early era, transcatheter atrial defect closure and AV dilations were associated with a low success rate (60% for both lesions) and a high complication rate (40% for atrial septal defect, 30% for AV dilations). Among the last 10 children, the atrial defect occlusion was successful in 100% without complications and AV dilations where successful in all children with a 30% complication rate (one major, two minor).
A learning curve with device development plays a significant role in the evolution of transcatheter techniques. These data provide baseline estimates of success and may be used as a template in the future when new techniques are adapted into practice.
Williams syndrome (WS) is a congenital, multisystem developmental disorder affecting 1 in 8000 live births. Cardiovascular abnormalities are present in 80% of WS patients. The present study sought to characterize fully the electrocardiographic findings in WS and correlate findings with anatomic pathology.
A retrospective review was performed of the electrocardiograms (ECGs) of patients with WS evaluated at the Children's Hospital of Philadelphia from January 1, 1980 through December 31, 2007. When available, the five most recent ECGs in each patient were evaluated. Ventricular hypertrophy was diagnosed based on previously reported voltage criteria in normal populations.
There were 187 patients with 499 ECGs for evaluation. Median age at study ECG was 8.0 years (range 0.1–58.9); median number of ECGs per patient was 2.7 (range 1–5). Heart rate, PR interval, and QRS interval were normal for age. Right ventricular hypertrophy (RVH) was present on 44% (219/499) of ECGs. Left ventricular hypertrophy (LVH) was present on 30% (150/499) of ECGs. Fifty-seven percent (106/187) of subjects had ≥1 ECG demonstrating RVH, and 39% (72/187) had ≥1 ECG demonstrating LVH. The severity of right- and left-sided obstructive lesions correlated with the ECG presence of RVH (P < .0001, odds ratio 21.8) and LVH (P < .001, odds ratio 14.5–61), respectively.
Electrocardiographic intervals in patients with WS follow expected trends seen in normal patients. Voltage criteria for RVH and LVH are commonly met on ECGs of patients with WS. The presence of ventricular hypertrophy on ECG in WS correlates with lesion severity.
The study aims to examine acute and midterm outcomes after percutaneous interventions for treatment of pulmonary artery stenosis (PAS) in patients with Alagille Syndrome (ALGS).
PAS affects up to two thirds of ALGS patients. Responsiveness to transcatheter therapies may differ from other causes of PAS. To date, there has been no study to evaluate outcomes of transcatheter interventions on PAS exclusively in ALGS.
In this single-center series, we reviewed procedural, hemodynamic, and angiographic data from patients with ALGS and PAS from 2007 to 2011 who underwent an interventional catheterization. Minimal luminal diameter (MLD) was assessed pre- and postintervention, and at follow-up catheterization(s) when available. Acute and midterm response to high-pressure balloon angioplasty (HBA), bare metal stent (BMS) placement, and cutting balloon angioplasty (CBA) were assessed.
Nine patients (median age 9.1 years) underwent 16 cardiac catheterizations with 34 interventions performed (20 HBA, 11 BMS, 3 CBA). There was a significant acute increase in MLD for all three modalities (42% HBA, P < .01; 91% BMS, P < .01; 58% CBA, P = .04). Follow-up data were available for 19 treated lesions at a median of 11 months. There was no significant difference in the improvement of MLD from baseline between the HBA and BMS groups, although in contrast to the BMS group, the HBA group showed continued interval vessel growth.
Transcatheter intervention for PAS in ALGS is generally safe and acutely effective. Although BMS implantation was associated with the greatest immediate improvement in MLD, HBA-treated vessels demonstrate interval growth, whereas BMS-treated lesions do not.
The objective of this study was to (1) describe the technical aspects of fluoroscopy-guided umbilical venous catheter placement (FGUVCP); and (2) determine the procedural success rate, factors contributing to procedural failure, and risks of the procedure.
Umbilical venous catheters are advantageous compared with femoral venous access, but can be difficult to place at the bedside.
This was a retrospective chart review from a single tertiary care referral institution.
FGUVCP was successful in 138 of 180 patients (76.7%) over a seven-year period. Patients in whom FGUVCP was successful were younger at the time of procedure compared with patients in whom FGUVCP was unsuccessful (median 18.2 vs. 22.2 hours, P = .03). The optimal age cutoff to predict FGUVCP success was 20 hours with a high positive predictive value (82.4%) but low negative predictive value (32.5%). No other variables were associated with procedural failure, though functional univentricular heart and older gestational age trended toward statistical significance. Median radiation time, contrast exposure, and blood loss were 3.2 minutes, 1 mL, and 1 mL, respectively. A total of 10 complications in 10 patients were associated with FGUVCP.
FGUVCP is a safe and highly successful way to obtain central venous access in neonates with congenital heart disease. Older age at the time of procedure is associated with procedural failure, but utilization of an age cutoff may not be clinically useful.
Diastolic dyssynchrony is increasingly being recognized as another marker for possible adverse cardiac events. Minimal data exist in hypoplastic left heart syndrome (HLHS) patients. The goal of this study was to determine if there were differences in diastolic dyssynchrony in patients with HLHS vs. control patients.
Tissue Doppler imaging (TDI) and strain rate (SR) analysis of the right ventricle in HLHS and control patients were performed. Time interval from onset of QRS complex on electrocardiography to peak TDI e′ wave was obtained. Differences in intervals were calculated: QRSe′(RV) − QRSe′(IVS) and QRSe′(RV) − QRSe′LV). Time interval from onset of QRS to peak strain rate early diastolic wave (SRe) was obtained for the six-segment model RV. Standard deviation of the six SRe time intervals was calculated. t-tests were performed to determine if differences were present between groups. Sixty patients were studied (35 HLHS, 25 control). There were no significant differences between HLHS and control patients in age (6.5 ± 3.2 years vs. 6.1 ± 2.6 years) or heart rate (91 ± 22 bpm vs. 91 ± 13 bpm), respectively. There were no significant differences between HLHS and control patients in QRSe′(RV) − QRSe′(IVS) (19.9 ± 15.4 ms vs. 23.3 ± 13.6 ms) and QRSe′(RV) − QRSe′(LV) (20.7 ± 13.8 vs. 22.8 ± 14.6 ms), respectively. There was a significant difference in SRe standard deviation between the HLHS and control patients (25.3 ± 12.4 ms vs. 15.5 ± 6.9 ms, P = .0007), respectively.
Patients with HLHS had increased diastolic dyssynchrony compared with control patients as measured via deformation analysis. Future studies are needed to determine the significance of these findings.
Neurocardiogenic syncope (NCS) is the most common cause of syncope in children and adolescents. Neurocardiogenic syncope occurs secondary to cerebral hypotension because of bradycardia, hypotension, or both. Head-up tilt-table test (HUTT) is the primary diagnostic test. Near-infrared spectroscopy (NIRS) is a noninvasive technology that directly monitors trends in regional tissue oxygen saturations over a specific body region. Placing an NIRS probe over the temporal region allows an indirect measurement of cerebral perfusion. Our hypothesis is that regional tissue oxygen saturation will decrease during an NCS episode and will remain stable in patients without syncope.
The investigators conducted a retrospective review of all HUTT utilizing cephalic NIRS performed at our institution from August 2012 to January 2013. Tests were classified as positive, negative, or psychogenic reactions. Paired t-test was used to determine statistical significance of NIRS changes and one-way analysis of variance was used to analyze baseline characteristics among the three groups.
Twelve patients were included in the study (female = 10). The average age was 14.4 years (range: 12–17). Five tests were positive for NCS, four were negative, and three demonstrated psychogenic reactions. Patients with a positive test had a sudden, significant decrease in regional tissue oxygen saturations (P = .009) by an average of 11.3 ± 5.2% compared with baseline. The decrease in regional tissue oxygen saturation preceded symptoms, hypotension, and bradycardia in all patients. Regional tissue oxygen saturation levels remained stable in patients with a negative test or psychogenic syncope.
NIRS monitoring during HUTT produces a reliable, positive result that precedes clinical signs and symptoms. Further, it helps distinguish NCS from psychogenic syncope.
To assess the effect of a multifaceted quality improvement intervention on variability of left ventricular dimensions as measured on pediatric echocardiograms.
Variability of measurements of left ventricular dimensions was assessed using percent error before a multifaceted educational intervention. Sonographers attended group sessions, used visual prompts and received individual feedback regarding the proper technique for measuring the left ventricle. Variability was reassessed following the intervention.
Sonographers were more likely to use proper measurement technique (38% vs. 62%, P = .017) after the educational intervention. The percent error of the left ventricular end systolic measurement decreased significantly (12.4% to 8.8%, P = .002). The percent error of the left ventricular end diastolic measurement showed a trend toward a decrease (7.7% to 5.8%, P = .067). There was no significant improvement in percent error for other measurements. Use of correct technique and sonographer experience of less than 5 years predicted decreased percent error.
A multifaceted educational intervention can be used to improve measurement variability in pediatric echocardiography laboratories.
After complete surgical repair the majority of tetralogy of Fallot (ToF), patients stay ≤2 days in the Cardiac Intensive Care Unit (CICU) while some may stay longer. We undertook this study to investigate the factors associated with shorter vs. longer length of stay in the CICU to help manage resources effectively.
Patients who underwent ToF repair at Aga Khan University, Pakistan, between July 2006 and December 2011 were studied in a case-control design. Clinical parameters were compared between short stay group (SSG) (≤2 days) and long stay group (LSG) (>2 days). Odds ratios were calculated, and regression was performed.
Ninety-eight patients (LSG 65, SSG 33) were included. Patients with lower preoperative saturation were 2.67 times more likely to be in the LSG group (P = .02). At 4 hours postoperatively, patients with a higher inotropic score (odds ratio [OR] = 3.03, confidence interval [CI] = 1.19–7.7, P = .02), higher central venous pressure (OR = 3.04, CI = 1.27–7.32, P = .013), and significant tachycardia at 4 hours (OR = 3.5, CI = 1.19–10.3. P = .02) were at risk for having a prolonged CICU stay. On multivariate analysis, significant postoperative tachycardia at 4 hours (z-score ≥3) was highly specific (sensitivity = 38.5%, specificity = 84.9%) for predicting the chances of being in the LSG. Other predictors included preop O2 saturation ≤82.5% (sensitivity = 61.1%, specificity = 63.0%) and CVP ≥10 mm Hg at 4 hours (sensitivity = 55.4%, specificity = 71.9%).
Patients who end up staying longer in the CICU have features that are distinctive in the immediate postoperative period, and this can help clinicians in identifying patients who may need more support.
Long QT syndrome (LQTS) is a genetic heart rhythm disorder that may present with syncope, seizures, or sudden cardiac death. Breath holding spells (BHS) occur in 5% of all children and have been noted in children with LQTS anecdotally. The purpose of this study was to determine the frequency of BHS in children diagnosed with LQTS at ≤5 years of age.
A retrospective review was performed to identify children diagnosed with LQTS who were ≤5 years old at initial presentation to our LQTS clinic from August 1999 to November 2013. The mean length of follow-up was 6.4 ± 2.8 years. The electronic medical records were reviewed for clinical presentation of BHS, as well as LQTS-associated symptoms, diagnostic tests, and treatment.
The study cohort consisted of 115 children with LQTS (58% male; median age at diagnosis, 11 months [range, birth to 5 years]; mean corrected QT interval (QTc), 478 ± 60 milliseconds). At presentation, 80% of patients were asymptomatic. Genetic testing revealed type 1 LQTS (LQT1) in 48%. Overall, 5 of 115 patients (4.3%) had BHS (2 of 5 [40%] male, mean QTc: 492 ± 14 milliseconds, 4 [80%] with family history of LQTS). BHS were the presenting symptom in 1 of 23 symptomatic patients (4.3%). All BHS occurred in patients with LQT1 (P = .02).
Although BHS among children with LQTS are relatively rare and occur at similar frequency as the general population, they can be the presenting symptom for a heart rhythm disorder. Careful attention to BHS is important to distinguish an innocent BHS from a potential LQTS-triggered cardiac event so that proper treatment is initiated.
Heart transplant recipients are at risk for developing left ventricular (LV) dysfunction. While traditional echocardiographic parameters have limitations, speckle tracking echocardiography (STE) is a novel technique shown to be more sensitive and accurate in adult studies for evaluating ventricular systolic and diastolic function.
Pediatric heart transplant recipients undergoing routine cardiac catheterization were included. Ratio of mitral early diastolic velocity-to-strain rate during early LV filling (E/SRE) and global longitudinal peak systolic strain (GLPSS) was measured by STE imaging. These were compared with wedge pressures by catheterization and traditional echocardiographic parameters.
A total of 50 subjects (46% males) were included. Mean age of the subjects was 13.0 ± 6.3 years and time since transplant was 4.1 years (range 0.2–17.1 years). While peak mitral inflow to late diastolic velocity (E/A ratio) was the only traditional diastolic function parameter having significant correlation with pulmonary capillary wedge pressure (PCWP) (r = 0.3; P = .3), STE-derived E/SRE had modest correlation with PCWP (r = 0.55; P < .01). Also, while most traditional systolic function parameters were normal, 12 subjects (24%) had GLPSS > −18%. Interestingly, subjects with coronary artery disease (n = 6) had significantly higher E/SRE (71.9 ± 28.4) compared to subjects without (45.2 ± 10.8; P < .001).
Diastolic function parameters by STE imaging correlate better with gold standard PCWP measurement than traditional echocardiographic parameters. Also, utilizing STE, abnormalities of longitudinal LV systolic function may be more common than previously thought in pediatric heart transplant recipients without acute graft rejection, despite "normal" systolic function by traditional echocardiogram.
The aim of the study was to determine right and left ventricle deformation parameters in patients with transposition of the great arteries who had undergone atrial or arterial switch procedures.
Patients with transposition are born with a systemic right ventricle. Historically, the atrial switch operation, in which the right ventricle remains the systemic ventricle, was performed. These patients have increased rates of morbidity and mortality. We used cardiac MRI with Velocity Vector Imaging analysis to characterize and compare ventricular myocardial deformation in patients who had an atrial switch or arterial switch operation.
Patients with a history of these procedures, who had a clinically ordered cardiac MRI were included in the study. Consecutive 20 patients (75% male, 28.7 ± 1.8 years) who underwent atrial switch operation and 20 patients (60% male, 17.7 ± 1.9 years) who underwent arterial switch operation were included in the study. Four chamber and short-axis cine images were used to determine longitudinal and circumferential strain and strain rate using Vector Velocity Imaging software.
Compared with the arterial switch group, the atrial switch group had decreased right ventricular ejection fraction and increased end-diastolic and end-systolic volumes, and no difference in left ventricular ejection fraction and volumes. The atrial switch group had decreased longitudinal and circumferential strain and strain rate. When compared with normal controls multiple strain parameters in the atrial switch group were reduced.
Myocardial deformation analysis of transposition patients reveals a reduction of right ventricular function and decreased longitudinal and circumferential strain parameters in patients with an atrial switch operation compared with those with arterial switch operation. A better understanding of the mechanisms of right ventricle failure in transposition of great arteries may lead to improved therapies and adaptation.
Deficient retro-aortic rim has been identified as a risk factor for device erosion following trans-catheter closure of atrial septal defects (ASDs). Transthoracic echocardiography (TTE) is the primary screening method for subjects for possible device closure of ASD, but its reliability in measuring retro-aortic rim size has not been assessed previously.
A single-institution cross-sectional analysis of children and adults referred for trans-catheter device closure of single ostium secundum ASD from January 1, 2005 to April 1, 2012 with reviewable TTE and trans-esophageal echocardiogram images was performed. Inter-rater reliability of measurements was tested in a 24% sample. Accuracy of TTE measurement of retro-aortic rim was assessed using a Bland–Altman plot with trans-esophageal echocardiogram measurement as the gold standard. Test characteristics of TTE detection of deficient retro-aortic rim were calculated. Risk factors for misclassification of deficient retro-aortic rim were assessed using receiver operator characteristic curves. Risk factors for measurement error were assessed through multivariate linear regression.
In total, 163 subjects of median age 5 years (range: 0.3–46 years) were included. Trans-thoracic echocardiography had 90% sensitivity, 84% specificity, 90% positive predictive value, and 83% negative predictive value to detect deficient retro-aortic rim. Bland–Altman plot demonstrated no fixed bias (P = .23), but errors in measurement increased on average as the aortic rim increased in size (P < .001). Prespecified patient level risk factors did not affect receiver operator characteristic curve area under the curve, nor were any patient-level risk factors independently associated with increased measurement error on TTE.
TTE is a sensitive and specific screening test for deficient retro-aortic rim across a range of patient ages and sizes.
Chylothorax complicates the postoperative course of patients after congenital heart surgery. Innominate vein thrombosis and stenosis have been associated with postoperative chylothorax. Revascularization and angioplasty can be accomplished using transcatheter techniques. We report our experience with this procedure for the management of postoperative chylothorax.
This is a retrospective case series of patients who underwent catheter revascularization and/or angioplasty of the innominate vein following cardiac surgery at our institution from January 1, 2008 through April 9, 2014.
The cardiovascular intensive care unit and cardiac catheterization laboratory at the University of Alabama at Birmingham and Benjamin Russell Hospital for Children in Birmingham, Alabama were used as settings for the study.
Out of 112 patients with postoperative chylothorax, 7 (6.3%) underwent transcatheter dilation of the innominate vein for occlusion/stenosis. The median age of the cohort was 1 month (15 days–6 years); median weight was 3 kg (2.7–22.2). Diagnosis was made a median 8 days (2–20) and persisted for a median of 24 days (9–44). Most patients failed medical management (low fat diet, nothing by mouth, and/or octreotide).
Cardiac catheterization occurred at a median 9 days (2–29) after chylothorax diagnosis. Median chest tube output on the day prior to procedure was 63 (12–149) cc/kg/day and decreased to 23 (0–64) cc/kg/day 2 days postprocedure (P = .01). Effusions resolved in a median of 5 days (1–16). There were no clinical complications postcatheterization. All patients who have undergone repeat angiography have maintained patency of the innominate vein. There was no mortality. Complications from chylothorax included prolong hospitalization, hyponatremia, hypoproteinemia, coagulopathy, lymphopenia, and infection.
Innominate vein occlusion and stenosis associated with chylous effusion are amenable to transcatheter revascularization and/or angioplasty, consistently leading to improvement, if not full resolution of chylothorax.
The electrocardiogram (ECG) is used to detect left ventricular hypertrophy (LVH) in children and young adults, and it is considered an important screening tool for the most common causes of sudden cardiac arrest in the United States. However, the reliability and accuracy of the ECG in the detection of LVH are poorly understood. This study's objective was to assess the reliability and accuracy of ECG parameters predicting LVH in comparison with echocardiographic two-dimensional left ventricular mass (2D-LVM) assessment.
Two hundred fifty patients met study criteria with complete 2D-LVM measurements, temporally matched ECGs and no confounding structural heart defects. The echocardiographic diagnosis of LVH was made by 2D-LVM z-score > 2. The electrocardiographic diagnosis of LVH was fulfilled if specific Q, R, or S wave voltages exceeded the normal limits for the respective age groups established by the Davignon, Macfarlane and Rijnbeek datasets, or fulfilled the LVH criteria set forth by adult indices (Sokolow–Lyon, Cornell, Gubner, Romhilt–Estes).
None of the ECG parameters showed significant correlation with 2D-LVM z-scores. In the detection of LVH, the most commonly used R in V6 voltages showed the following results: sensitivity 13–29%, specificity 77–96%, positive predictive value (PPV) 29–50%, and negative predictive value (NPV) 77%. The overall accuracy ranged 65–75%. The accuracy of adult indices for LVH ranged from 49% to 78%.
The ECG's prediction of LVH is unreliable with dismal sensitivity, variable specificity, poor PPV, and barely acceptable NPV. With such overall poor reliability, the use of current ECG standards in screening for LVH warrants reassessment.
Catheter ablation of the slow atrioventricular (AV) pathway has been shown to be safe and effective in pediatric patients with atrioventricular nodal reentrant tachycardia (AVNRT). Despite that, acute success is not guaranteed, and safety of ablating near the AV node remains a concern.
A retrospective analysis was performed of all AVNRT ablations using the Ensite NavX voltage mapping technique at our institution. Each map was reviewed with patient and NavX computer data recorded. To account for a learning curve, each map was idealized and compared with the original map. Procedure and fluoroscopy time were compared with a control group.
Twenty-eight patients underwent catheter ablation for AVNRT from September 2011 until December 2012 using the voltage mapping technique. The historical control group comprised 24 patients who underwent catheter ablation using the electroanatomic approach. There was 96% acute success with one recurrence in the voltage mapping group, at a mean follow-up of 24 months. The slow pathway was visualized in 86% of patients at the time of ablation, while three of four without could be found on idealization of the voltage map. Mean high- and low-voltage parameters increased with idealization, but showed no correlation with age, gender, or weight. Estimated pathway size had significant inter-patient variability. Procedure and fluoroscopy times did not vary significantly compared with controls.
Visualization of the AV nodal slow pathway in a pediatric population is possible using voltage mapping technique with the potential to increase safety and efficacy. Variability exists in the voltage parameters needed to visualize individual slow pathways, which leads to a distinct learning curve.
Acute kidney injury (AKI) is a common complication resulting from cardiopulmonary bypass in infants. Urinary neutrophil gelatinase-associated lipocalin (NGAL) is a sensitive and specific marker of such injury. In this study, we compared the performance of serum cystatin C (Cys C) and serum creatinine (Cr) as early markers of renal dysfunction in infants undergoing cardiac surgery under bypass.
The study was designed as a prospective observational study. The study was conducted in the cardiac intensive care unit (ICU) of a tertiary, academic children's hospital in the United States. Infants (age <1 year) undergoing cardiac surgery under cardiopulmonary bypass were included in the study.
Acute kidney injury was defined based on postoperative urinary NGAL.
A total of 17 infants were included in the study, and five of them developed AKI. Serum Cys C and Cr levels were measured postoperatively on days 1, 2, and 3, and compared with baseline levels. On postoperative day 2, infants with AKI showed significant change from baseline in serum Cys C levels compared with non-AKI infants (28% vs. −9%, P = .03). The two groups did not show significant differences with respect to rise in serum Cr on any of the 3 postoperative days. Serum Cr on days 1 and 2 showed nonspecific increases in both AKI and non-AKI groups. The area under the receiver operating characteristic curve for day 2 Cys C was 0.87 (95% CI 0.67–1.00) in recognizing NGAL-positive AKI.
Postoperative serum Cys C appears to be a more specific and sensitive biomarker for NGAL-positive AKI resulting from cardiopulmonary bypass surgery in infants undergoing cardiac surgery.
Ventricular assist devices (VADs) have emerged as an important treatment option for bridging pediatric patients with heart failure to transplant. VADs have shown improved survival; however, the pediatric quality of life (QoL) while on VAD support is unknown. We aimed to evaluate the QoL of our pediatric patients while supported with a VAD.
In this prospective study, pediatric patients who underwent VAD placement, and their parents, were administered a generic Pediatric Quality of Life Inventory (PedsQL) 4.0 pre-VAD implant, when feasible, after the acute postoperative period, and then periodically until heart transplant or death. Their final scores while on support were compared with three previously reported groups: healthy controls, outpatients with severe heart disease, and children after heart transplant.
From January 2008 to July 2014, 13 pediatric patients required VAD support greater than 2 weeks and completed a PedsQL. The mean age at implant was 10.0 ± 4.2 years and median duration of support was 1.6 (0.5–19.7) months. Eleven (85%) patients survived to transplant with one (8%) patient remaining alive on support. The median duration of support prior to their final PedsQL was 1.4 (0.5–11.4) months. Patients self-reported significantly (P < .05) lower total and physical QoL scores when compared with all three comparison groups. Self-reported psychosocial QoL scores were significantly lower than healthy controls only. Parent proxy-reported scores were significantly lower than all three comparison groups for all three categories (P < .05).
A large deficit exists in the total QoL of pediatric patients supported by a VAD compared with outpatient management of severe heart disease or postheart transplant patients; however, VAD patients do represent a group with more severe heart failure. Improvements in QoL must be made, as time spent with a VAD will likely continue to increase.


text:
Tom Ladshaw wrote:Earlier in his career (before hitting on the magic theme), Manuel was known as "Manuel, Master of the Mighty Colon".
Tom Ladshaw wrote: The "Conjurer's Commode" was Ben Martin's variation on Ed Marlo's seminal "Cardician's Crapper". Which in itself was highly derivative of Vernon's "Thaumaturgist's Toilet" (described in the expurgated edition of Hugard & Braue's "Expert at the Card Table" as Charlie Miller's "Prestidigitator's Pooper"). Vernon got the notion from Larry Grey, the "Dizzy Wizard"....with his "Wizard's Wizzer". No idea where Larry Grey got it from...though there's speculation that he cribbed it from "Manuel, Master of the Mighty Dollar". Earlier in his career (before hitting on the magic theme), Manuel was known as "Manuel, Master of the Mighty Colon". The less said about that act, the better....though I *will* say it was a solid knockoff of LePetomane's classic act.**
I'm sorry. I can't resist potty humor.
Mr. Ash! Calling Mr. Ash!
** It's rumored that Josef Pujols ("LePetomane") always claimed that when compared to other acts, his was a breeze...
Sorry...I just couldn't leave well enough alone...
Rene Clement wrote:
Briefly, A card is selected. The magician opens this wooden box and takes out a small vaudville-like eisel and cards that read "Presenting", etc.... then take out a doll house commode. You have a small roll of toliet paper and tweezers. You reach into the commode with the tweezers and remove this brown mini-poo. Open it (it is really a plastic pill capsule under the brown puddy)and inside is a tiny playing card duplicate of the choosen card.

subset: commoncrawl


text:
U.S. productivity fell in the first quarter while labor costs rose, reflecting the economic slowdown caused at the start of the year by harsh winter weather.
U.S. productivity fell in the first quarter while labor costs rose, reflecting the economic slowdown caused at the start of the year by harsh winter weather.
The Labor Department said Wednesday that productivity declined at an annual rate of 1.7 percent in the January-March period, after growing at a 2.3 percent rate in last year's fourth quarter.
"Special factors, namely the weather, are to blame," said Jennifer Lee, a senior economist at BMO Capital Markets.
The falling productivity coupled with a slight increase in hourly compensation led to labor costs rising 4.2 percent in the first quarter. Labor costs had fallen in the previous two quarters.
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Economic growth stalled in the first three months of this year, increasing just 0.1 percent, according to initial estimates by the Commerce Department. Freezing temperatures and snow storms disrupted growth. Retail sales and factory output plunged in January, only to recover as the weather warmed.
The economy might even have shrunk 0.6 percent during the first quarter, the research firm Marcoeconomic Advisers said Tuesday after the release of March trade data.
Productivity measures output per hour of work. Greater productivity should raise living standards because it enables companies to pay their workers more without having to increase prices, which could boost inflation.
Despite the increase in labor costs last quarter, overall inflation remains mild.
The Federal Reserve tracks productivity and labor costs when setting interest rate policies. Fed officials have attempted to spur growth by keeping short-term interest rates at record lows, while at the same time, purchasing bonds to try to keep long-term rates down as well.
Since December, however, the Fed has reduced its monthly bond-buying to $45 billion from $85 billion.
But the Fed is committed to holding down short-term rates for an extended period, saying that it expects to maintain those rates "well past" the time that unemployment dipped below its previous threshold of 6.5 percent.
The unemployment rate fell in April to 6.3 percent from 6.7 percent, a sign that economic growth should rebound in the second quarter.